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Polycythemia Vera (PV): Update on Emerging Treatment Options

Authors Benevolo G, Vassallo F, Urbino I, Giai V

Received 7 January 2021

Accepted for publication 21 February 2021

Published 16 March 2021 Volume 2021:17 Pages 209—221


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Professor Garry Walsh

Giulia Benevolo, Francesco Vassallo, Irene Urbino, Valentina Giai

Division of Haematology, Città della Salute e della Scienza, Turin, Italy

Correspondence: Giulia Benevolo
Hematology, Città della Salute e della Scienza, Corso Bramante 88/90, Turin, 10126, Italy
Email [email protected]

Abstract: Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by exuberant red cell production leading to a broad range of symptoms that compromise quality of life and productivity of patients. PV reduces survival expectation, primarily due to thrombotic events, transformation to blast phase and post-PV myelofibrosis or to development of second cancers, which are associates with poor prognosis. Current therapeutic first line recommendations based on risk adapted classification divided patients into two groups, according to age (< or > 60 years) and presence of prior thrombotic events. Low-risk patients (age < 60 years and no prior history of thrombosis) should be treated with aspirin (81– 100 mg/d) and phlebotomy, to maintain hematocrit < 45%. High-risk patients (age > 60 years and/or prior history of thrombosis), in addition to aspirin and phlebotomies, should receive cytoreductive therapy in order to reduce thrombotic risk. Nowadays hydroxyurea still remains the cytoreductive agent of first choice, reserving Interferon to young patients or childbearing women. During the last years, ruxolitinib emerged as a new treatment in PV patients, as second line therapy: it appeared especially effective in patients with severe pruritus, symptomatic splenomegaly, or post-PV myelofibrosis symptoms. Currently, in PV treatment, several molecules have been tested or are under investigation. At present, the drug that has shown the most encouraging results is givinostat.

Keywords: polycythemia vera, therapy, ruxolitinib, interferon, givinostat

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